2002
DOI: 10.1590/s0004-282x2002000500026
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Abstract: A paralisia bulbar progressiva, também denominada doença de Fazio-Londe, caracteriza-se pelo acometimento degenerativo progressivo de nervos cranianos bulbares em crianças. Foi descrita primeiramente por Fazio em 1892 e até a presente data somente 30 casos foram relatados na literatura. Acomete ambos os sexos, assumindo dois padrões clínicos, um de início precoce (idade <6 anos, predomínio de comprometimento respiratório) e outro de início tardio (6-20 anos, predomínio de comprometimento motor nos membros s… Show more

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Cited by 3 publications
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“…PBP can be divided into childhood-and adult-onset forms [6]. The childhood-onset form, also called Fazio-Londe disease, is an autosomal recessive disease associated with progressive impairment of the cranial nerves with two clinical subtypes-an early course, typically starting before the age of 6 years with mainly respiratory symptoms, and a late course starting between 6 and 20 years with mainly motor symptoms in the upper limbs [7]. The adult-onset form attacks the bulbar region and clinically presents with swallowing, speaking, and chewing difficulties [8].…”
Section: Introductionmentioning
confidence: 99%
“…PBP can be divided into childhood-and adult-onset forms [6]. The childhood-onset form, also called Fazio-Londe disease, is an autosomal recessive disease associated with progressive impairment of the cranial nerves with two clinical subtypes-an early course, typically starting before the age of 6 years with mainly respiratory symptoms, and a late course starting between 6 and 20 years with mainly motor symptoms in the upper limbs [7]. The adult-onset form attacks the bulbar region and clinically presents with swallowing, speaking, and chewing difficulties [8].…”
Section: Introductionmentioning
confidence: 99%
“…A Paralisia Bulbar Progressiva (PBP) é um distúrbio neurológico, também conhecido como paralisia glosso-labio-laringea, e descrito atualmente como paralisa bulbar, devido a sua localização de lesão anatômica, existente na parte superior da medula espinhal (Robinson, 1878). É uma doença rara, degenerativa e progressiva, que atinge ambos os sexos (Batista et al, 2002). Com predomínio no sexo feminino (Chieia, 2005).…”
Section: Introductionunclassified